A Case Report: Recurrence of Vogt-Koyanagi-Harada Disease after Rapid Tapering of Corticosteroids

Abstract

Background: Vogt-Koyanagi-Harada's disease (VKHD) is an autoimmune disorder that targets melanocyte-rich tissues in genetically susceptible individuals. It can affect the eyes, inner ears, skin, hair, and meninges. When VKHD affects the eye, it can cause bilateral granulomatous panuveitis with exudative bullous retinal detachment and is usually associated with meningismus. It may lead to permanent loss of visual acuity. While the pathogenesis remains unknown, viral infection has been implicated. The most accepted theory remains a T-cell mediated autoimmune reaction against one or more antigens associated with melanocytes, melanin, and retinal pigment epithelium (RPE).

Case presentation: We report the case of a 17-year-old male patient presenting to our outpatient department for vision loss. The patient reported a 2-week history of hearing loss, tinnitus, and episodic headaches associated with vomiting. Physical examination showed white lashes (poliosis) and a bilateral decrease in visual acuity. Fluorescein angiography showed bullous exudative retinal detachments. The patient was diagnosed with VKHD. His symptoms resolved following a course of oral prednisolone that was initiated at 60 mg per day (1mg/Kg/day), then tapered down over 6 weeks. Six months later, the patient presented again with more pronounced symptoms. His Best Corrected Visual Acuity (BCVA) was lower than it was on his first presentation, and the retinal exam showed a sunset glow fundus and degenerative changes in the RPE. Combined therapy with prednisolone and methotrexate resulted in a complete resolution of symptoms, and his BCVA returned to more than 20/40.

Conclusion: This case emphasizes the importance of avoiding the rapid tapering of corticosteroids in VKHD due to the high risk of disease recurrence.